Ausgewählte seltene rhinologische Krankheitsbilder Pathogenese – Klinik – Therapie

Zusammenfassung

Seltene rhinologische Krankheitsbilder sind zunächst eine diagnostische Herausforderung. Von der Primärmanifestation bis zur definitiven Diagnose vergehen meist Monate bis Jahre in denen der Krankheitsprozess unkontrolliert oder insuffizient behandelt fortschreitet. So entstehen (irreversible) Schäden und teils lebensbedrohliche Situationen. Der unerwartete Verlauf einer (fehldiagnostizierten) Erkrankung sollte zu weiteren diagnostischen Überlegungen und Schritten führen, um auch die seltenen Erkrankungen frühzeitig zu erkennen.
In der vorliegenden Arbeit werden granulomatöse Erkrankungen der Nase und Nasennebenhöhlen hervorgerufen durch Mykobakterien, Treponemen, Klebsiellen, Pilze und Protozoen sowie Vaskulitiden, die Sarkoidose, die Rosazea, die Kokain-induzierten Mittelliniendestruktionen, das nasale extranodale NK/T-Zellen-Lymphom und das Cholesterin Granulom diskutiert. Des Weiteren werden Krankheitsbilder mit Störungen der mukoziliären Clearance wie die primären ziliären Dyskinesien und die Mukoviszidose unter Berücksichtigung der aktuellen Literatur besprochen.

Abstract

Rare rhinological diseases are a diagnostic challenge. From the primary manifestation until the final diagnosis it may take several months or even up to years in which the disease progress is uncontrolled and the disease insufficiently treated. This results in (irreversible) damage and possibly life-threatening situations. The unexpected course of a (misdiagnosed) disease should lead to further diagnostic considerations and steps to detect the orphan disease at a stage as early as possible. In the present work granulomatous diseases of the nose and sinuses caused by mycobacteria, treponemes, klebsiella, fungi and protozoa as well as vasculitis, sarcoidosis, rosacea, the cocaine-induced midline destruction, the nasal extranodal NK/T-cell lymphoma and cholesterol granuloma are discussed. Furthermore diseases with disorders of mucociliary clearance as the primary ciliary dyskinesia and cystic fibrosis are presented taking into account the current literature.

Schlüsselwörter

Nase - Nasennebenhöhlen - seltene Erkrankung

Key words

nose - paranasal sinuses - orphan disease - rare disease

• Literatur

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